Rheum D/O 4 Flashcards
triple symptoms of Bahcet’s dz
recurrent uveitis
oral aphthous ulcers
genital ulcers
where is Bahcet’s dz MC
ancient Silk Road
easter EU to the Mediterranean
Turkish, Chinese, Japanese, Korean, Saudi Arabian
Bahcet’s dz patho
infectious pathogen triggers a hyperactive immune response in genetically susceptible person
possible heat shock proteins driving the reaction
proposed infectious agents of Bahcet’s dz
herpes simplex Strep Staph H. Pylori E. coli
Bahcet’s dz levels of immunity affected
B and T cells
autoantibody production
activation of coagulation cascade
vessels Bahcet’s dz
vasculitis lesions of small, medium, and larger arteries and veins
VESSELS of ALL SIZES
epidemiology Bahcet’s dz
25-35 yrs
Bahcet’s dz spares
liver
kidneys
heart
clinical manifestations
- oral ulcers
- genital ulcers
- cutaneous lesions
- ocular disease
- neurologic dz
- arterial dz
Bahcet’s dz oral ulcers
canker sores
more extensive than typical
multiple, painful. limit eating
heal 1-3 weeks+
most specific sign for Bahcet’s dz
genital ulcers
painful, heal with scarring, most often on scrotum/vulva
cutaneous lesions (list)
Acneiform pathergy dermaographism erythema nodosum pyoderma hangrenosum
acneiform Bahcet’s dz
arthritis, looks like regular acne
pathergy Bahcet’s dz
formation of papule 48 hrs after skin prick
cutaneous reaction following skin stimulation
dermographism
light scratching produces pathology
erythema nodosum
medium vessel vasculitis that ULCERATES
Bahcet’s dz making diagnosis
3 episodes of oral herpetiform or apthous ulceration in 12 months (observed or reported)
2 recurrent/painful genital ulcers and ophthalmic lesions
and
2 skin lesions or positive potherb skin testing
pharm tx Bahcet’s dz
gdlucocritcoids colchicine (mucocutaneous dz) azathiprine severe immunosuppressive agents TNF Inhibitors
polymyositis
idiopathic autoimmune inlammatory myopathy
- symmetrical, proximal muscle weakness
- elevated CK levels
- characteristic EMG and muscle bx findings
dermatomyositis is different from polymyositis
due to associated rash
dermatomyositis polymyositis epidemiology
specific agents
MC in African American
2x as common in women
dermatomyositis: children 5-14, adults 50+
polymyositis: adults, 45-60s
etiology polymyositis
altered CELLULAR immunity
T cell mediated cytotoxic process against Ag on myocytes
associated dz polymyositis
MC with other autoimmune dz
dermatomyositis etiology
altered HUMERAL immunity
directed against muscle capillaries and small arteriole
complement activation = mircoinfarction
dermatomyositis polymyositis association
underlying malignancy infection medicaitons rheumatology disease HLA subtypes
infection of dermatomyositis polymyositis
HIV
coxsackievirus
HBV
influenza
dermatomyositis polymyositis presentation
symmetrical and proximal painless myopathy
weakness of large muscles of hip and shoulder girdles develop over 3 - 6 months
weak neck muscles of neck flexion = difficult to hold head up
sensation and DTRs preserved
dysphagia
constitutional symptoms
interstitial lung disease
dermatomyositis pathognomonic
heliotrope rash
dermatomyositis polymyositis work up
- CPK (elevated 5-50x)
- EMG abnormal
- muscle biopsy
- malignancy screening
dermatomyositis polymyositis tx (direct dz)
initial tx corticosteroids (prednisone) 1mg/kg/day x6-8 weeks and tapered
typically responds well, but if not give cytotoxic
vitamin D and Ca for osteoporosis
dermatomyositis polymyositis CA
incidence of lung, bladder, non-Hodgkin lymphoma are all increased in 1st year after diagnosis
prognosis dermatomyositis polymyositis
mortality is associated to malignancy and pulmonary complication
5 yr survival >80%
inclusion body myositis
idiopathic autoimmune inflammatory myopathy
more common in men, >50
proximal and distal muscles affected
